CD56-negative extranodal nasal type NK/T-cell lymphoma
ABSTRACT Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare lymphoma that occurs predominantly in Asian adults. In this report, we describe the clinical and pathologic features of an unusual aggressive lymphoid neoplasm in a child and review the literature on NK/T-cell lymphoma in children. The patient was a 4-year-old Native American male with facial swelling, lymphadenopathy, and fevers. Biopsy demonstrated neoplastic lymphoid cells that expressed CD3, CD8, TIA-1, and EBV-encoded RNA without CD56. The patient failed multiagent chemotherapy and died of therapy-related complications. This case represents an extranodal NK/T-cell lymphoma, nasal type, with an unusual lack of CD56.
Pediatric Hematology and Oncology 03/2012; 29(2):166-9. DOI:10.3109/08880018.2011.639044 · 0.96 Impact Factor
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ABSTRACT: BACKGROUND: Hydroa vacciniforme (HV)-like lymphoma (HVL) is a rare and aggressive cutaneous T-cell lymphoma occurring mainly in children in Latin America and Asia. Chronic latent Epstein-Barr virus infection has been associated with both HV and HVL. OBJECTIVE: We sought to evaluate the clinical presentation and histopathology of this rare cutaneous T-cell lymphoma. METHODS: We reviewed the clinical, morphologic, and immunophenotypical features in 12 cases of HVL from Bolivia. RESULTS: All 12 patients had skin lesions in both sun-exposed and nonsun-exposed areas, including edema, blistering, ulceration, and scarring, with a slowly progressive relapsing course. All 12 patients presented with systemic symptoms and showed a characteristic swelling of the nose and lips, and periorbital edema. Eight patients died an average of 5.3 months after initial diagnosis. Four patients remained alive with persistent disease. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity. The immunophenotype showed a cytotoxic T-cell (CD8(+)) profile. All cases were associated with Epstein-Barr virus infection and differed clinically from other forms of cutaneous T-cell lymphoma. LIMITATIONS: Only a limited number of cases were studied. CONCLUSIONS: This study confirms that HVL is a highly aggressive lymphoma, although some patients have a more indolent, chronic course.Journal of the American Academy of Dermatology 03/2013; 69(1). DOI:10.1016/j.jaad.2013.01.037 · 5.00 Impact Factor
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ABSTRACT: Primary lymphoma of adrenal glands is rare, and non-B-cell lymphoma associated with pyothorax is also very rare. Here we report the first autopsy case of non-B-cell lymphoma in bilateral adrenal glands of a 79-year-old woman with pyothorax who had an aggressive clinical course. Immunohistochemically, tumor cells showed CD3+, CD45RO+, CD5-, CD7-, CD4-, CD8-, CD10-, CD20-, CD30-, CD79a-, CD138-, CD56-, granzyme B-, TIA-1+ and ALK-. In addition, tumor cells were strongly EBER1-positive by in situ hybridization. In genomic DNA of tumor cells, T-cell receptor rearrangements were not detected by southern blotting. We finally diagnosed this case as extranodal NK/T-cell lymphoma (nasal type). Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8050621197741854.Diagnostic Pathology 08/2012; 7(1):114. DOI:10.1186/1746-1596-7-114 · 2.41 Impact Factor